Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan.

ABSTRACT:

Rationale

Acquired pulmonary alveolar proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation occurring in association with granulocyte/macrophage colony-stimulating factor autoantibodies (autoimmune PAP) or as a consequence of another disease (secondary PAP). Because PAP is rare, prior reports were based on limited patient numbers or a synthesis of historical data.

Objectives

To describe the epidemiologic, clinical, physiologic, and laboratory features of autoimmune PAP in a large, contemporaneous cohort of patients with PAP.

Methods

Over 6 years, 248 patients with PAP were enrolled in a Japanese national registry, including 223 with autoimmune PAP.

Measurements and main results

Autoimmune PAP represented 89.9% of cases and had a min

SUBMITTER: Inoue Y 

PROVIDER: S-EPMC2720118 | biostudies-literature | 2008 Apr

REPOSITORIES: biostudies-literature